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Uptodate Reference Title
WHO diagnostic criteria overt primary myelofibrosis
WHO diagnostic criteria overt primary myelofibrosis
Major criteria
Presence of megakaryocytic proliferation and atypia, accompanied by either reticulin and/or collagen fibrosis grades 2 or 3
Not meeting WHO criteria for ET, PV, BCR-ABL1+ CML, myelodysplastic syndromes, or other myeloid neoplasms
Presence of JAK2, CALR, or MPL mutation or, in the absence of these mutations, presence of another clonal marker,* or absence of reactive myelofibrosis¶
Minor criteria
Presence of at least 1 of the following, confirmed in 2 consecutive determinations:
Anemia not attributed to a comorbid condition
Leukocytosis ≥11 × 109/L
Palpable splenomegaly
LDH increased to above upper normal limit of institutional reference range
Leukoerythroblastosis
Diagnosis of overt PMF requires meeting all 3 major criteria, and at least 1 minor criterion
CML: chronic myeloid leukemia; ET: essential thrombocythemia; LDH: lactate dehydrogenase; PMF: primary myelofibrosis; PV: polycythemia vera * In the absence of any of the 3 major clonal mutations, the search for the most frequent accompanying mutations (eg, ASXL1, EZH2, TET2, IDH1/IDH2, SRSF2, SF3B1) are of help in determining the clonal nature of the disease. ¶ Bone marrow fibrosis secondary to infection, autoimmune disorder, or other chronic inflammatory conditions, hairy cell leukemia or other lymphoid neoplasm, metastatic malignancy, or toxic (chronic) myelopathies.